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Publication Exclusive: Taking a closer look at persistent hyperplastic primary vitreous
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Persistent hyperplastic primary vitreous, or PHPV, is a form of persistent fetal vasculature, first described by Reese in 1955. The exact etiology of PHPV remains unknown, but it characteristically presents as unilateral leukocoria. PHPV is divided into subtypes: anterior, posterior and combined. In anterior PHPV, the remnant vascular stalk is seen attached to the posterior surface of the lens capsule, but it does not extend back to the optic nerve. Posterior PHPV occurs when the remnant vascular stalk is seen arising off the optic nerve but does not reach the lens. Posterior PHPV may be associated with developmental abnormalities of the optic nerve and retina. In the combined type, features of both anterior and posterior PHPV are often detected, and they give a mixed presentation.
Clinical features and pathology
Patients with PHPV present with varied clinical features depending on the extent of involvement of the ocular structure due to non-regression of fetal vasculature or a persistent hyaloid artery. They usually present with unilateral, rarely bilateral, leukocoria. Leukocoria is often the presenting feature, and it can result due to persistence of fibrovascular structure behind the lens; cataract; persistent dense pupillary membrane; or cloudy cornea secondary to raised IOP.
Children may present with a shallow anterior chamber and raised IOP secondary to the presence of fibrovascular tissue in ectopic locations due to the formation of peripheral anterior synechiae, posterior synechiae and a shift of the lens-iris diaphragm anteriorly. In PHPV, the fetal vasculature may invade the lens and cause a lenticular hemorrhage if perfusion persists. This eventually leads to cataract formation that can vary from partial to complete.
Patients may also present with decreased vision, microphthalmia, strabismus and amblyopia. Amblyopia may be a presenting symptom in PHPV due to a severe but previously undiscovered isolated posterior abnormality without an obvious anterior component.
Retinal detachment is often encountered with epiretinal fibrovascular tissue. When PHPV is associated with macular traction, degeneration and epiretinal membrane, there is usually severe ocular morbidity. The optic nerve may also be hypoplastic or dysplastic.
Click here to read the full publication exclusive, Complications Consult, published in Ocular Surgery News U.S. Edition, September 10, 2016.